293 pathogenesis of barrier syndrome 294 further

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293
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PATHOGENESIS OF BARRIER SYNDROME 294 Further activates the renin angiotensin axis
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CLINICAL FEATURE OF BARTTER’S SYNDROME CLASSIC BARTTER’S SYNDROME PRESENTS DURING CHILDHOOD WEAKNESS AND CRAMPS OCCUR SECONDARY TO HYPOKALEMIA POLYURIA AND NOCTURIA ARE COMMON DUE TO HYPOKALEMIA . NEPHROCALCINOSIS (DUE TO HYPERCALCIURIA) 295
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DIAGNOSIS OF BARTTER’S SYNDROME: HYPO KALEMIA . METABOLIC ALKALOSIS . BLOOD PRESSURE IS USUALLY NORMAL . URINARY POTASSIUM, CALCIUM AND SODIUM ELEVATED. SERUM RENIN, ALDOSTERONE AND POSTAGLANDIN E ELEVATED. RENAL FUNCTION IS TYPICALLY NORMAL. 296
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297
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GITTEMAN’S SYNDROME AUTOSOMAL RECESSIVE TRAIT CAUSED DUE TO MUTATION IN THIAZIDE SENSITIVE NA-CL TRANSPORTER. LOSS OF ACTIVITY OF THE THAZIDE SENSITIVE TRANSPORTER INCREASES TUBULAR CALCIUM REABSORPTION LEADING TO CLASSIC FINDING OF HYPOCLACIURIA IN GITLEMAN’S SYNDROME. THERE IS ALSO DECREASE IN NA+ REABSORPTIONE LEADING TO VOLUME DEPLETION AND HYPOKALEMIA. 298
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299
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ALL OF THE FOLLOWING STATEMENTS ABOUT BARTTER SYNDROME AND GITELMAN’S SYNDROME ARE TRUE EXCEPT- (NBE/DNB PATTERN) A) AUTOSOMAL RECESSIVE INHERITANCE B) BARTTER SYNDROME PRESENTS EARLIER IN LIFE THAN GITELMAN’S SYNDROME C) GENETIC DEFECT BARTTER SYNDROME INVOLVES THE TRANSPORT PROTEINS IN THE DISTAL TUBULE D) HYPERCALCIURIA IS MORE COMMON IN BARTTER SYNDROME ANS. IS ‘C’ I.E., GENETIC DEFECT IN BARTTER SYNDROME INVOLVES THE TRANSPORT PROTEINS IN THE DISTAL TUBULE . 300
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302
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ALL OF THE FOLLOWING ARE FEATURES OF BARTTER’S SYNDROME, EXCEPT - (NBE/DNB PATTERN) A) HYPOKALEMIA B) HYPERMAGNESEMIA C) HYPERPROSTAGLANDINE D) HYPER CALCIUREA ANS. IS ‘B’ I.E., HYPERMAGNESEMIA [REF: HARRISON 18,H/EP. 2360 & L?H/EP. 1801] 303
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MUTATION IN ALPHA 5 CHAIN OF COLLAGEN 4, THE DIAGONISIS - (AIIMS NOV 06) A) ALPORT’S SYNDROME B) THIN MEMBRANE DISEASE C) NODULAR GLOMERULOSCLEROSIS D) GOOD PASTURE SYNDROME 304
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ALPORT’S SYNDROME IS A HEREDITARY NEPHRITIS, CHARACTERIZED BY - MICROSCOPIC HEMATURIA (FIRST SYMPTOM). - DEAFNESS. PATHOLOGY OF ALPORT’S SYNDROME ALPORT’S SYNDROME IS CHARACTERIZED BV DIFFUSE GLOMERULAR BASEMENT MEMBRANE THICKENING. THE BASEMENT MEMBRANE OF THE GLOMERULUS IS MADE UP OF TYPE IV COLLAGEN. 305
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306
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PATHOGENESIS OF GOOD PASTURE SYNDROME GOOD PASTURE SYNDROME IS AN UNCOMMON AUTOIMMUNE DISEASE, CHARACTERIZED BY THE PRESENCE OF CIRCULATING AUTOANTIBODIES TARGETED AGAINST THE NONCOLLAGENOUS DOMAIN OF THE ALPHA- 3 CHAIN OF COLLAGEN IV. 307
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308
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INDICATION OF DIALYSIS IS ALL EXCEPT- A) PERICARDITIS (NEET/DNB PATTERN) B) PERISTENT HYPERKALEMIA C) UREMIC ENCEPHALOPATHY D) SEVERE VOLUME OVERLOAD 309
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310
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CONTRAINDICATIONS FOR DIALYSIS RECENT ABDOMINAL AND/OR CARDIOTHORACIC SURGERY DIAPHRAGMATIC PERITONEAL-PLEURAL CONNECTIONS SEVERE RESPIRATORY FAILURE LIFE-THREATENING HYPERKALEMIA EXTREMELY HIGH CATABOLISM SEVERE VOLUME OVERLOAD IN A PATIENT NOT ON A VENTILATOR 312
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